A case and review of Haemoglobin SE in the paediatric population.
HbSE is a rare hemoglobinopathy with conflicting reports in the
literature regarding potential complications. We report an 11-year old
girl with HbSE disease and resultant bone infarction most likely due to
vaso-occlusion. She had similar HbS and HbE levels as her sister, who
has had no complications to date. We performed a literature review and
identified a further 23 pediatric cases of Hemoglobin SE and although
the majority of patients remained asymptomatic, up to 50% of children
may suffer mostly vas-occlusive complications, some resulting in death.
Infection and hyposplenism were uncommon. Further reports and case
series of HbSE are needed to clarify the natural history of this rare